Overview

"Little's disease" redirects here.

Spastic diplegia is a neuromuscular type of cerebral palsy involving hypertonia and spasticity in the muscles of the lower extremities, usually those of the legs, hips and pelvis. Doctor William John Little's first recorded encounter with cerebral palsy is reported to have been among pediatric patients who displayed signs of spastic diplegia; for this reason, spastic diplegia was widely known as Little's Disease for many years, and some clinicians continue to use this term.

This condition is by far the most common type of CP, occurring in almost 70% of all cases.

Causes

Spastic diplegia is mainly congenital; after-birth causes, such as exposure to toxins, traumatic brain injury, encephalitis, meningitis, drowning or suffocation are miniscule by comparison. Instead, neonatal asphyxia, hypoxia of the brain, premature birth, birth trauma, hematoma in the brain, or the presence of certain maternal infections during pregnancy are all the primary forms of brain damage at birth that can prevent the proper development of the pyramidal tract, the motor cortex or corticospinal tract. If such areas of the brain are affected, affected nerve receptors in the spine are in turn unable to properly absorb the gamma amino butyric acid (GABA) which would otherwise properly regulate tone in the affected areas. Without GABA, affected nerves perpetually fire the message for their corresponding muscles to contract and tighten up. This results in perpetual tightness and stiffness in movement for the person.

Although certainly not the only cause of the spastic diplegia form of cerebral palsy, periventricular leukomalacia is a rather common underlying factor.

Presentation

The degree of spasticity in spastic diplegia, and, for that matter, other types of spastic CP, can and almost always does vary widely from person to person. No two people with spastic diplegia are exactly alike, and no form of CP is genetic, nor has any reliable scientific research yet been done to point to susceptibility to CP along hereditary lines.

Above the hips, persons with spastic diplegia typically retain normal or near-normal muscle tone and range of motion, though some lesser spasticity may also affect the upper body, such as the trunk and arms, depending on the severity of the condition in the individual. Additionally, because leg tightness often leads to instability in ambulation, extra muscle tension usually develops in the upper body, shoulders, and arms due to compensatory stabilization movements, regardless of the fact that the upper body itself is not directly affected by the condition. Meanwhile, lower-extremity spasticity in spastic diplegia is rarely so great as to totally prevent ambulation — i.e., most people with the condition can walk — but the main difference between spastic diplegia and a normal gait pattern is that spastic diplegia does result in the signature "scissor gait" that some able-bodied people might tend to confuse with the effects of drunkenness, multiple sclerosis or another nerve disease.

Terminology

Socially, it should be noted that although the term "spastic" technically describes the attribute of spasticity in spastic cerebral palsy and was originally an acceptable and common term to use in both self-description and in description by others, it has since gained more notoriety as a pejorative, particularly when used in pop culture to insult able-bodied people when they seem overly anxious or unskilled in sports (see also the article spazz). In 1952 a UK charitable organization with a membership mainly of those with spastic CP was formed; this organization called itself The Spastics Society. However, the charity changed its name to Scope in 1994 due to the term spastic having become enough of a pejorative to warrant the name change.

Treatment

Muscle stretching, range of motion exercises, yoga, contact improvisation, modern dance, resistance training, and other physical activity regimens are often utilized by those with spastic CP to help prevent contractures and reduce the severity of symptoms. Some places within the United States have also issued medical marijuana to help treat spasticity.

Major clinical treatments for spastic diplegia are:

baclofen (and its derivatives), a gamma amino butyric acid substitute injected into the spinal fluid for trial, and thereafter administered either orally or via an intrathecal pump;

phenol, injected selectively into the over-firing nerves in the legs on the muscle end to reduce spasticity in their corresponding muscles;

orthopedic surgery to release the spastic muscles from their hypertonic state, a usually temporary result because of the source of the spasticity being in the nerves, not the muscles; and,

Rhizotomy, a neurosurgery directly targeting and eliminating (cutting) the over-firing nerve rootlets and leaving the properly-firing ones intact, thereby permanently eliminating the spasticity but compelling the person to re-strengthen muscles that will have been severely weakened by the loss of the spasticity.